Abstract
Angiomyolipomas are benign mesenchymal neoplasms, presenting with a variable mixture of adipose tissue, smooth muscle and vascular component. Although they are typically found in the kidneys, many cases of extrarenal angiomyolipomas have been reported. They are extremely rarely present in the uterus. We describe a case of a 56-year-old woman, operated on for leiomyoma. Total laparohysterectomy and bilateral adnexectomy was performed. After histological and immunohistochemical examination, the final diagnosis of uterine angiomyolipoma was made. Renal and extrarenal angiomyolipomas are compared in regard to clinical and morphological aspect and their difference from PEComas is dicussed. PEComas have been defined during the last decade and there are still issues regarding terminological clarity and overlapping.